Case Report/Caso Clínico
72 Acta Obstet Ginecol Port 2018;12(1):72-74
INTRODUCTION
S
quamous cell carcinoma (SCC) of the vulva is an uncommon disease accounting for 4% of all gynae- cological cancers1. In Portugal, the incidence rate in 2010 was 3.2/100000 women2. The disease typically develops in older women and is rarely seen in patients younger than 35 years of age1. Recently, an increase in the incidence of vulvar SCC in young women has been observed1,3,4, and besides the known association with human papillomavirus (HPV) infection, immune sup- pression conditions and tobacco use have also been im- plicated as predisposing factors in the development of SCC in this age group3-6.Attempting an early diagnosis is the key, since the 5-year survival in early stages (FIGO I-II) is high (>80%), falling to less than 50% if the inguinal nodes
*Assistente hospitalar no Hospital de Braga
**Interna de formação específica de Ginecologia e Obstetrícia do Hospital de Braga
***Assistente Hospitalar Graduada, Sub-especialista e Mestre em Ginecologia Oncológica
are involved and 10–15% if the iliac or other pelvic no- des are positive4. Additionally, in the young sub-group of patients, the often mutilating local treatment requi- red in advanced stages of the disease has more poten- tial mental trauma associated, and this psichological aspect should not be minored.
CASE REPORT
A nulliparous 28-year-old caucasian female presented to the gynecologic clinic harboring a positive cervical co-test: high-grade squamous intraepithelial lesion (HSIL) plus high-risk HPV positive (excluding HPV-16 and HPV-18). She mentioned a painless vulvar lesion for the past six months. Her past and family history were irrelevant, except for tobacco use (5 cigarettes/
/day). She refered 21 as the age of sexual initiation and two sexual partners since then. The contraceptive me - thod was the combined oral contraceptive pill. The physical examination was relevant for a 8mm vulvar ulcer, localized lateral to the posterior fourchette(Fi- gure 1); the cervix was macroscopically normal and
Abstract
Although the incidence of preinvasive vulvar lesions seems to be rising in the younger population, invasive squamous cell carcinoma remains exceedingly rare. As a result, diagnostic biopsy is often delayed and ablative therapy is frequently ins- tituted without an adequate histologic diagnosis.
We present an invasive squamous carcinoma of the vulva in a 28-year-old nulliparous woman who presented with a pain- less vulvar ulcer for the past 6 months. Biopsy revealed an invasive squamous cell carcinoma of the vulva. Conservative surgery (tumorectomy with safety margins and sentinel lymph node biopsy) was performed with good patient recovery and return to normal working life before 30 days.
Keywords:Vulvar cancer; Premenopausal; Human papillomavirus infection.
Carcinoma of the vulva in young women:
a diagnosis to be kept in mind Carcinoma da vulva na mulher jovem:
um diagnóstico a ter em mente
Catarina Pardal*, Natacha Sousa**, Cátia Correia*, Carla Monteiro*, Paula Serrano***
Serviço de Ginecologia e Obstetrícia do Hospital de Braga
Catarina Pardal et al.
Acta Obstet Ginecol Port 2018;12(1):72-74 73
the colposcopy was adequate for evaluation, with a squamo-columnar junction not visible, a transforma- tion zone type 3 and no abnormal findings. The vagi- na had normal colposcopic findings, and vulvoscopy revealed no additional lesions. Punch biopsy of the vulvar ulcer revealed a specimen (1.2 cm) with HSIL and SCC with stromal invasion > 1 mm (cFIGO >=IB) (Figure 2). In the diagnostic assessment, essential and aquired immunossupressive disorders were excluded and the patient underwent surgical staging with dou- ble ipsilateral sentinel lymph node technic. Lympho- cintigraphy was performed the day prior to surgery and showed two right inguinal nodes (‘hot’ nodes).
The intra-operative peri-tumoral injection of metile- ne-blue marked the same 2 nodes (‘blue’ nodes) with frozen biopsy negative for carcinoma. Conservative vulvar surgery with radical tumorectomy with appa- rent safety margins was performed plus large loop ex- cision of transformation zone. The patient had an un - eventfull postoperative and was discharged 3 days la- ter. The definitive histologic assessment revealed a vulvar SCC (pT1bG2N0 (SN) – FIGO IB) with 1 cm free margin, and in the cervical specimen only LSIL was present.
The patient is in her first year of follow-up (Col- poscopy Unit and Gynaecologic Oncology Unit), with satisfatory surgical esthetical results, no complaints of dispareunia or vulvodynia and with no disease recur- rence to date.
DISCUSSION
Vulvar cancer is rare, especially in very young patients with most of the published data presented as case re- ports. By age group, the incidence trend of vulvar SCC has remained relatively stable over the last three deca- des, although the incidence in women aged 40–49 years has risen two-fold4. This increase has been re- flected in many reports from several countries1,3,4and has been ascribed to the effect of increasing HPV in- fection7, particularly types 16, 18 and 33. Despite the known HPV infection pathogenesis in genital carcino- ma, other predisposing factors such as depressed im- munity have been implicated in the progression of VIN/HSIL to invasive disease at an earlier age. Factors affecting a patient’s immunity include genetic factors, oncogenic virus exposure, medical conditions (ex. dia- betes, lupus), immunossupressive or cytotoxic che- motherapy, as well as exposure to carcinogenic subs- tances. Smoking has been implicated in the develop- ment of the invasive genital cancers, and it appears to lead to humoral immune suppression and such im- mune response appears to be responsible for reactiva- tion of viroses remaining latent in tissues6. This preci- pitates a local immunodeficiency, allowing the patient to be susceptible to other co-factors which predispo- se to the development of dysplasia or intraepithelial neoplasia and subsequent invasive cancer.
Despite the presence of several risk factors that increase the odds of developing vulvar cancer, most women do not develop it, and some women who don’t have any apparent risk factors develop vulvar cancer. In our pa-
FIGURE 1. Vulvar ulcer.
FIGURE 2. HE100X. Squamous cell carcinoma, moderately differentiated
Carcinoma of the vulva in young women: a diagnosis to be kept in mind
74 Acta Obstet Ginecol Port 2018;12(1):72-74
tient, both high risk HPV infection and smoking status were pesent. However, young patients with these risk factors and a competente immune system, rarely pre- sent with invasive neoplasia. Other factors not fully un- derstood might be implied in the carcinogenesis of the- se neoplasias. An interesting fact is that the cervical le- sion detected in the transformation zone specimen, which is the most susceptible area to HPV infection and associated intra-epithelial neoplasia, was of lesser se- verity than the vulvar lesion. And also, that the disea- se was focal, rather than with the typical multifocal pat- tern of usual VIN. However, vulvoscopy has low sen- sitivity for VIN, and other lesions might have been pre- sent without colposcopic detection, meaning a long period of close follow-up is of most importance in the- se patients. A conservative approach, such as the one described in the case report, is the treatment of choice in this subpopulation, but obtaining a disease-free mar- gin of at least 1 cm is the most important prognostic fac- tor for recurrent disease. Particularly in this young po- pulation, the avoidance of radical surgery which is as- sociated with major distortion of normal vulvar anato- my, is fulcral for the woman’s self-esteem.
Young women with vulvar SCC do not appear to have more advanced disease when first diagnosed or to have a different course of the disease compared with their elderly counterparts. However, in this subgroup of patients the development of other genital carcinomas later in life is more frequent (new primary lesions ra - ther than recurrence of the original vulvar carcinoma) and an increased awareness of cervical, vaginal and vul- var symptoms are mandatory.
The optimal follow-up schedule for vulvar cancer is undetermined, but the European Society of Gynaeco- logic Oncology recommend clinical examination of vulva and groins every three-four months in the first two years, biannually in the third and fourth year, and anual long-term follow-up afterward8. Currently, our patient is in surveillance in the Colposcopic Unit and
Gynaecologic Unit every 3 months period, with no re- current disease. Because of the high risk of recurrence and other lower genital dysplasia/neoplasia lesions, the patient was encouraged to perform self-examination and report any suspicious lesions encoutered at any time.
Although rare, vulvar SCC in young women is a pos- sibility with potentially life-threatening implications and is certainly a diagnosis to be kept in mind.
REFERENCES
1. Neville F. Hacker, Patricia J. Eifel, Jacobus van der Velden.
FIGO cancer report 2015. International Journal of Gynecology and Obstetrics 131 (2015) S76–S83.
2. RORENO. Registo Oncológico Nacional 2010. Instituto Por- tuguês de Oncologia do Porto Francisco Gentil – EPE, ed. Porto, 2016.
3. Al-Ghamdi A., Freedman D., Miller D., et al. Vulvar Squa- mous Cell Carcinoma in Young Women: A Clinicopathologic Stu- dy of 21 Cases. Gynecologic Oncology 2002, 84, 94–101.
4. RCOG Guidelines for the Diagnosis and Management of Vul- val Carcinomas. https://www.rcog.org.uk
5. Linn Woelber, Fabian Trillsch, Lili Kock et al. Management of patients with vulvar cancer: a perspective review according to tu- mour stage. TherAdv Med Oncol. 2013 May; 5(3): 183-192.
6. Carter J, Carlson J, Fowler J, et al. Invasive vulvar tumors in young women—a disease of the immunossuppressed?. Gynecol On- col 1993;51:307–10.
7. Cancer Research UK. Vulval cancer incidence statistics http://www.cancerresearchuk.org/cancer-info/cancerstats/types/vul- va/incidence/
8. ESGO. Vulvar Cancer Guidelines. https://www.esgo.org/wp- content/uploads/2016/10/ESGO-Vulvar-cancer-Complete-re- port.pdf
ENDEREÇO PARA CORRESPONDÊNCIA Catarina Pardal
Hospital de Braga Braga, Portugal
E-mail: catarinapardal_84@hotmail.com RECEBIDO EM: 23/98/2017
ACEITE PARA PUBLICAÇÃO: 19/10/2017
